Amyloidosis of the larynx.

Amyloidosis of the larynx is an uncommon disease and mainly a local occurrence. Hoarseness is the prevalent symptom. Surgical excision of the amyloid masses is the treatment of choice. In the present paper, the authors describe seven cases treated in the last twenty years.

beta-Hydroxybutyrate decreases adenosine triphosphate degradation products in human subjects.

Many disease states decrease intracellular adenosine triphosphate (ATP) levels and elevate body fluid purine levels. The use of specific metabolic substrates may reverse this process. This study was designed to test the hypothesis that beta-hydroxybutyrate, a substrate for ATP synthesis, decreases body fluid purine levels during interventions that induce ATP degradation. Decreases in these purine levels are metabolic markers for diminished ATP degradation. Two human models for stimulating ATP degradation were used to test the hypothesis. Rapid fructose infusion causes acute degradation of hepatic ATP, and ischemic exercise stimulates ATP consumption in skeletal muscle. The activity of beta-hydroxybutyrate was used in combination with phosphate, another important substrate for ATP synthesis. The studies were performed during a low-phosphate state in 10 normal subjects and during a high-phosphate state in 7 normal subjects. Metabolic variables, such as serum or urinary phosphate level, blood beta-hydroxybutyrate level, blood acetoacetate level, plasma or urinary purine level, blood lactate level, and blood ammonia level, were monitored during the study. After ischemic exercise of the forearm muscle, beta-hydroxybutyrate decreased the level of plasma total purines, blood lactate, and blood ammonia during the low-phosphate state but not during the high-phosphate state. During fructose-induced hepatic ATP breakdown, beta-hydroxybutyrate decreased late phase plasma purine increases under low-phosphate conditions only and decreased urinary total and radiolabeled purine elevations under both phosphate conditions. These data indicate that the infusion of beta-hydroxybutyrate may alter the balance from ATP degradation toward ATP resynthesis in muscle and liver by providing an immediate source of fuel and reducing equivalents under under specific metabolic conditions. This activity in combination with other metabolic interventions may have therapeutic value by restoring ATP pools in ATP-depleted tissues.

Altered properties of human T-lymphoblast soluble low Km 5'-nucleotidase: comparison with B-lymphoblast enzyme.

Soluble low Km 5'-nucleotidases have been purified from human cultured T- and B-lymphoblasts to compare their properties and to examine the mechanism of different rates of nucleotide dephosphorylation. The enzyme from B-lymphoblasts (MGL-8) was 4385-fold purified with a specific activity of 114 mumol/min/mg, while the enzyme from T-lymphoblasts (CEM, MOLT-4) was 4355-fold purified with a specific activity of 35 mumol/min/mg. The activity of both enzymes have an absolute requirement for Mg++. The B-cell enzyme has maximum activity with Mg2+ > Mn2+ > Co2+, while the T-cell enzyme had maximum activity with Co2+ > Mn2+ > Mg2+. The optimum activity was at pH 7.4-9.0 for the B-cell enzyme and pH 9.0 for the T-cell enzyme. Substrate specificity was the same for both enzymes with the following relative Vmax values: CMP > UMP > dUMP > dCMP > dAMP > IMP > GMP > dIMP > dGMP. The Km values for AMP and IMP were 12 and 25 microM for the B-cell enzyme, and 7.0 and 12 microM for the T-cell enzyme. ATP and ADP are competitive inhibitors of these enzymes with apparent Ki values of 100 and 20 microM for the B-cell enzyme, and 44 microM and 8 microM for the T-cell enzyme, respectively. The apparent molecular mass by gel filtration column chromatography is 145 kD for the B-cell enzyme and 72 kDa for the T-cell enzyme. The subunit molecular masses by Western blots are 69.2 kD for both enzymes. These properties suggest that the B-lymphoblast enzyme is identical or similar to the enzyme from human placenta. However, the T-cell enzyme has some different properties. We conclude that these differences plus a lower content of low Km 5'-nucleotidase in T-cells may account for the decreased ability of T-lymphoblasts to dephosphorylate nucleotides and may contribute to the selective cytotoxicity of deoxyribonucleosides for T-lymphoblasts as compared to B-lymphoblasts.

The significance of some immunoserologic diagnostic tests in systemic diseases.

The sensitivity and specificity of immunoserologic tests included in the diagnostic criteria of systemic lupus erythematosus are shown. The frequencies of some rare autoantibodies (PCNA, PL-4, SL, PM-Scl, Scl-70, Jo-1) in systemic connective tissue diseases of mixed Yugoslav population are demonstrated. The results from different laboratories differ considerably and the possible reasons are described.